Health condition
Rotor and Dubin-Johnson syndromes
Rotor and Dubin-Johnson syndromes are rare, benign inherited causes of raised conjugated bilirubin - their importance lies in being recognised so they are not confused with genuine liver or bile-duct disease.
Why trust this guide
- 01
Editorial team
Written by our editorial team from the UK sources listed on each page.
- 02
Sourced from guidance
Checked against current NHS and UK specialist society guidance at the time of review.
- 03
Current for 2026
Reviewed on 2026-09-27 with the next review scheduled for 2027-09-27.
Key facts
The essentials at a glance.
Which gene and test confirm it, who should be tested in the family, and which specialist to see.
-
Both are autosomal recessive and cause raised conjugated (direct) bilirubin with otherwise normal liver tests
-
Dubin-Johnson involves ABCC2 variants; Rotor involves SLCO1B1 and SLCO1B3
-
Neither shortens life nor damages the liver; no treatment is required
-
Genetic testing can now confirm the diagnosis without liver biopsy
Understanding the condition
What it is and what happens next.
What causes it, how it is confirmed, and the testing and specialist routes available in the UK.
What it is and how it presents
Most inherited jaundices raise unconjugated bilirubin, but these two raise the conjugated fraction, the pattern doctors normally associate with bile-duct obstruction or liver injury, which is precisely why they cause diagnostic confusion. Affected people have mild fluctuating jaundice, sometimes noticed only during pregnancy, illness or when starting the contraceptive pill, with normal enzymes and imaging.
Testing, surveillance and specialist care
Historically the diagnosis needed urine porphyrin studies or a biopsy showing the characteristic dark pigment of Dubin-Johnson; today, sequencing ABCC2 or the SLCO1B1/1B3 genes settles it cleanly, and a hepatologist can confirm that no surveillance or treatment is needed. One caveat: Rotor-type transporter variants can alter the handling of certain drugs, including some statins. NHS clinical genetics services carry some of the longest waits in the health service, often many months for a first appointment, whereas private genetic testing and a specialist review can typically be arranged within around two weeks.
Seeing a specialist
Who to see, and how quickly you can be seen.
The specialist for this condition is usually a hepatologist. Your GP can refer you on the NHS, or you can arrange a private consultation directly.
A quiet reminder
This guide is for information, not medical advice.
Your GP knows your history and can tell you which parts apply to you. If in doubt, see them - and seek urgent care for any emergency symptoms.
Frequently asked
Common questions about Rotor and Dubin-Johnson syndromes.
Quick, plain-English answers to what people ask us most.
-
What are Rotor and Dubin-Johnson syndromes?
Rotor and Dubin-Johnson syndromes are rare, benign inherited causes of raised conjugated bilirubin in the blood - their main clinical importance lies in being recognised so they aren't mistaken for more serious liver disease.
-
What causes Rotor and Dubin-Johnson syndromes?
Both are inherited genetic conditions affecting how the liver processes and excretes bilirubin, causing mildly raised levels in the blood without any underlying liver damage or disease.
-
What are the symptoms of Rotor and Dubin-Johnson syndromes?
Most people have mild, intermittent yellowing of the skin or eyes (jaundice), often noticed incidentally, without any other symptoms of liver disease.
-
Are Rotor and Dubin-Johnson syndromes dangerous?
No, they are benign conditions that do not cause liver damage or long-term health problems, though the finding of raised bilirubin can cause unnecessary worry or investigation if not recognised as one of these conditions.
-
How are Rotor and Dubin-Johnson syndromes diagnosed?
Diagnosis involves specific blood tests to characterise the pattern of bilirubin elevation, sometimes alongside imaging or genetic testing, and crucially, excluding other causes of liver dysfunction.
-
Do Rotor and Dubin-Johnson syndromes need treatment?
No specific treatment is needed, as these are benign conditions; the main value of diagnosis is reassurance and avoiding unnecessary further liver investigation once confirmed.
-
Rotor and Dubin-Johnson syndromes vs Gilbert's syndrome - what's the difference?
Gilbert's syndrome causes raised unconjugated bilirubin and is very common and harmless, while Rotor and Dubin-Johnson syndromes cause raised conjugated bilirubin and are rarer, but similarly benign once correctly identified.
-
When should I see a doctor about raised bilirubin?
See your GP if a blood test shows raised bilirubin, so the specific pattern can be characterised and a benign cause distinguished from more concerning liver conditions. A private hepatologist can typically be seen within about two weeks if needed.